Korean J Hematol 2005; 40(1):
Published online March 30, 2005
https://doi.org/10.5045/kjh.2005.40.1.58
© The Korean Society of Hematology
박선영, 김진석, 김유리, 박인혜, 정준원, 이승태, 한지숙, 민유홍
연세대학교 의과대학 내과학교실
Acquired Hemophilia A is a rare and considerably life-threatening coagulopathy, which is caused by the development of autoantibodies against factor VIII (FVIII) in non-hemophilic adults. Acquired FVIII inhibitors can be associated with diverse conditions, such as malignant disorders, medications, autoimmune diseases, postpartum states and others. These autoantibodies inhibit normal coagulation, had results in bleeding complications, which can contribute to mortality in a high percentages of cases. Effective control of the disorder can be achieved by prompt diagnosis and appropriate managements. Generally, the managements of acquired hemophilia A are aimed at treating the acute bleeding and eliminating inhibitors by immunosuppression. Although a range of treatment options exists for patients with acquired hemophilia A, there is no consensus with regard to the optimal therapies for this disorder. Herein, two cases, an 82-year-old man and a 78-year-old man who were successfully treated by steroid or danazol, which is a relatively mild immunosuppressive agent, are reported.
Keywords Acquired hemophilia A, Factor VIII inhibitor, Immunosuppressive therapy
Korean J Hematol 2005; 40(1): 58-62
Published online March 30, 2005 https://doi.org/10.5045/kjh.2005.40.1.58
Copyright © The Korean Society of Hematology.
박선영, 김진석, 김유리, 박인혜, 정준원, 이승태, 한지숙, 민유홍
연세대학교 의과대학 내과학교실
Sun Young Park, Jin Seok Kim, Yuri Kim, In Hae Park, June, Won Cheong, Seung Tae Lee, Jee Sook Hahn, Yoo Hong Min
Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
Acquired Hemophilia A is a rare and considerably life-threatening coagulopathy, which is caused by the development of autoantibodies against factor VIII (FVIII) in non-hemophilic adults. Acquired FVIII inhibitors can be associated with diverse conditions, such as malignant disorders, medications, autoimmune diseases, postpartum states and others. These autoantibodies inhibit normal coagulation, had results in bleeding complications, which can contribute to mortality in a high percentages of cases. Effective control of the disorder can be achieved by prompt diagnosis and appropriate managements. Generally, the managements of acquired hemophilia A are aimed at treating the acute bleeding and eliminating inhibitors by immunosuppression. Although a range of treatment options exists for patients with acquired hemophilia A, there is no consensus with regard to the optimal therapies for this disorder. Herein, two cases, an 82-year-old man and a 78-year-old man who were successfully treated by steroid or danazol, which is a relatively mild immunosuppressive agent, are reported.
Keywords: Acquired hemophilia A, Factor VIII inhibitor, Immunosuppressive therapy
Hyun Ju Kim, Won Sik Lee, Young Jin Lee, Hyun Soo Jun, Su-Kil Seo, and Young-Don Joo
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